Figure 1

Clinical scoring of 25 CLN3 patients homozygous for the 1-kb deletion in the CLN3 gene. Scoring for each problem category was made as follows: normal function (3); problem slight, but readily recognized (2); problem severe (1); total loss of function (0). The range between the 10th and 90th percentile is represented by the shaded area. The black line represents the median.